Ductus Arteriosus Research Articles

Congenital heart disease: types, pathophysiology, diagnosis, and treatment options.

Congenital heart disease (CHD) is a structural abnormality of the heart and/or great vessels and patients with CHD are at an increased risks of various morbidities throughout their lives and reduced long-term survival. Eventually, CHD may result in various complications including heart failure, arrhythmias, stroke, pneumonia, and sudden death. Unfortunately, the exact etiology and pathophysiology of some CHD remain unclear. Although the quality of life and prognosis of patients with CHD have significantly improved following technological advancement, the influence of CHD is lifelong, especially in patients with complicated CHD. Thus, the management of CHD remains a challenge due to its high prevalence. Finally, there are some disagreements on CHD among international guidelines. In this review, we provide an update of the pathophysiology, diagnosis, and treatment in most common type of CHD, including patent foramen ovale, atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus, coarctation of the aorta, transposition of the great arteries, congenitally corrected transposition of the great arteries, coronary anomalies, left and right ventricular outflow tract obstruction, tetralogy of Fallot and Ebstein anomaly. In particular, we focus on what is known and what is unknown in these areas, aiming to improve the current understanding of various types of CHD.

Persistent right aortic arch with aberrant left subclavian artery originating from the patent ductus arteriosus in a dog: a case report

Persistent right aortic arch with aberrant left subclavian artery originating from the patent ductus arteriosus in a dog: a case report

PATENT DUCTUS ARTERIOSUS STENTING IN NEWBORNS AND INFANTS WITH CYANOTIC CONGENITAL HEART DEFECTS: EXPERIENCE AND OUTCOMES

Background. Among all congenital heart defects (CHD), one-third are cyanotic defects with duct-dependent pulmonary blood flow. Palliative interventions are necessary for children with complex cyanotic CHD, premature infants, and children with complex comorbidities. Ensuring a reliable source of pulmonary blood flow adequate for arterial oxygenation and native pulmonary artery branches growth are the primary goals of staged interventions. The classic approach to providing adequate pulmonary blood flow before total repair is systemic-to-pulmonary artery shunting. Currently, there is a growing trend towards the use of an alternative method - transcatheter patent ductus arteriosus (PDA) stenting. Aim: Demonstrate the experience of PDA stenting, immediate and long-term outcomes in newborns and infants. Materials and Methods. From 2009 to April 2024, PDA stenting was performed in 66 patients at the UCC. Among the all cohort, 49 (74.2%) were newborns (Group I), and 17 (25.7%) were infants (Group II). The average age was: Group I - 7.4±5.9 days (range 1 to 28 days), and Group II - 105.2±54.78 days (range 42 to 280 days). The average weight was: Group I - 3.0±0.49 kg (range 1.9 to 4.4 kg), and Group II - 5.2±1.4 kg (range 2.7 to 9 kg). This study analyzed pre-, intra-, and postoperative parameters including age, weight, presence of concomitant/genetic pathology, saturation level, pulmonary artery branches diameter, Nakata index, PDAs size, origin and type, duration of staying in the intensive care unit, duration of artificial lung ventilation and inotropic support, early and late postoperative complications, 30-day and inter-stage mortality rates in the two patient groups. Results. All cases of transcatheter stent implantation were performed by transfemoral access. The average ICU stay duration for Group I was 13.8±9.52 days (range 2 to 119 days), and for Group II was 6.4±3.64 days (range 2 to 20 days). The duration of artificial lung ventilation in Group I was 185.8±165.28 hours (range 2 to 2066 hours), and in Group II was 67.5±46.15 hours (range 4 to 248 hours). Inotropic support - on average for Group I - 151.6±81.06 hours (range 0 to 720 hours), and for Group II - 82.4±47 hours (range 0 to 211 hours). Saturation increased from an average of 65.7±11.03% to 85.2±5.7% in Group I (p<0.05), and from 66.8±10.9% to 87.4±4.24% in Group II (p<0.05). Early postoperative complications were observed in 34.6% (17/49) of Group I patients and 23.5% (4/17) of Group II patients. The early 30-day mortality rate was 8.1% (4/49) for Group I. The next stage of surgical treatment wasperformed on average after 12.9±10.2 months by 51% (25/49) of Group I patients and by 88% (15/17) of Group II patients, on average after 14.98±12 months post-stenting. The interstage (late) mortality rate was 6.1% (3/49) for Group I and 5.8% (1/17) for Group II. Conclusion. Considering the outcomes after PDA stenting in our cohort of patients, this procedure appears to be an effective operation for providing a reliable source of pulmonary blood flow in various anatomical variants of cyanotic CHD in both newborns and infants.

Narrative Review on Echocardiographic Evaluation of Patent Ductus Arteriosus in Preterm Infants.

Persistent Patent Ductus Arteriosus (PDA) is prevalent among extremely preterm infants, with its occurrence inversely related to gestational age. A persistent PDA correlates with increased mortality and morbidities such as intraventricular hemorrhage, pulmonary hemorrhage, chronic lung disease, bronchopulmonary dysplasia, and necrotizing enterocolitis as observed clinically. Conversely, numerous randomized controlled trials have failed to demonstrate significant benefits from PDA treatment. One contributing factor to these conflicting findings is that PDA affects each individual differently depending on the cardiovascular decompensation and its hemodynamic impact. PDA management should be based on the hemodynamic significance, rather than just the presence or size of PDA. This comprehensive narrative review paper describes echocardiographic parameters that allow a better understanding of the hemodynamic impact of PDA. A newer modality, like lung ultrasound, is also described here as an adjunct to assess the PDA impact on the lungs from pulmonary overcirculation.

Ізольована гіпоплазія правого шлуночка: діагностика, лікування та досвід спостереження пацієнтів дитячого віку

Isolated hypoplasia of the right ventricle (IHRV) is a rare congenital heart defect characterized by a reduction of the right ventricular cavity due to hypoplasia of its trabecular part. Aim - to analyze the anatomical variants of IHRV and their influence on the choice of treatment tactics based on the experience of observing pediatric patients. Materials and methods. This retrospective study included 11 consecutive patients diagnosed with IHRV from 2012 to 2024. Patients were divided into 3 groups based on the ratio of RV to left ventricle (LV) length. The Group I (n=4): mild IHRV (RV/LV length ratio >0.8). The Group II (n=4): moderate IHRV (RV/LV length ratio = 0.7-0.8). The Group III (n=3): severe IHRV (RV/LV ventricle length ratio <0.67). Results. From the Group I (n=4), one patient required medication therapy with beta-blockers and others did not require any treatment. From the Group II (n=4), one patient underwent Glenn shunt and patent foramen ovale (PFO) closure, another - Glenn shunt and PFO closure with fenestration. Two patients have stable clinical conditions. Patients from the Group III (n=3) had duct-dependent pulmonary blood flow in the neonatal period. One patient received treatment with beta-blockers; the second patient underwent patent ductus arteriosus stenting; and the third - systemic-to-pulmonary shunt. Conclusions. Patients with severe IHRV may have critical hemodynamic disturbances requiring urgent cardiac surgical interventions, whereas patients with mild and moderate hypoplasia may require medical treatment with possible surgical intervention at a later age. Long-term follow-up in this defect reveals a slow progressive growth of the right heart chambers. The research was carried out in accordance with the principles of the Declaration of Helsinki. Informed consent of the child and child's parents was obtained for the research. The authors declare no conflict of interest.

Transcatheter Cardiac Interventions in Neonates with Congenital Heart Disease: A Single Centre Experience

Objective: To analyze the outcome among neonates undergoing transcatheter balloon atrial septostomy for various kinds of congenital heart diseases. Study Design: Prospective longitudinal study. Place and Duration of Study: National Institute of Cardiovascular Diseases, Karachi Pakistan, from Feb 2020 to May 2021. Methodology: A total of 28 neonates of both genders with echocardiography proven diagnosis of Tricuspid Atresia with restrictive Patent Foramen Ovale, Dextro-Transposition of Great Arteries with Intact Ventricular Septum, complex cyanotic congenital heart disease with inadequate mixing or hypoplastic left heart syndrome with restrictive PFO and a non-significant Patent Ductus Arteriosus, undergoing transcatheter balloon atrial septostomy were included. Demographic data along with diagnosis, clinical presentation, echocardiographic findings, procedural outcome (successful/unsuccessful), duration of the procedure, complication (if any), post-procedural echocardiographic findings, duration of hospital stay, further treatment plan and 3-months follow up outcomes were recorded among all neonates. Results: Most common diagnosis was Dextro-Transposition of the Great Arteries with IVS observed in 14(50.0%) neonates. Procedural outcome was found to be successful among 23(82.1%) neonates. There were 19(67.9%) neonates who did not show any post-procedure complication. At 3-months follow up, improvement in blood saturation and weight gain were reported among 12(42.9%) neonates each. Overall, mortality was noted in 7(25.0%) neonates, of whom 4(57.1%) neonates were having hypoplastic left heart syndrome. Conclusion: Transcatheter atrial septostomy done in neonatal period was found to have positive outcomes. Mortality remains high among neonates undergoing transcatheter atrial septostomy having hypoplastic left heart syndrome.

Navigating the Challenges of Neonatal and Premature Patent Ductus Arteriosus Closure through Cardiac Surgical and Transcatheter Innovations

Navigating the Challenges of Neonatal and Premature Patent Ductus Arteriosus Closure through Cardiac Surgical and Transcatheter Innovations

Percutaneous closure of a large persistent ductus arteriosus in a pediatric patient with inferior vena cava interruption: a case report

Percutaneous closure of a large persistent ductus arteriosus in a pediatric patient with inferior vena cava interruption: a case report

Retrograde cerebral embolism and pulmonary embolism caused by patent ductus arteriosus: a case report

BackgroundAlthough rare, paradoxical embolism sometimes occurs with patent ductus arteriosus (PDA). This study presents a case of PDA-associated paradoxical embolism with acute ischemic stroke (AIS) and pulmonary embolism (PE) following thoracoscopic surgery.Case PresentationA 65-year-old woman developed acute-onset aphasia and right hemiparesis on the third day following thoracoscopic resection for a right lung tumor. Brain magnetic resonance imaging revealed multiple infarcts, and lower extremity venous Doppler ultrasound revealed deep vein thrombosis. The patient subsequently developed dyspnea, tachycardia, and hypoxemia. PE was confirmed by percutaneous transfemoral venous selective pulmonary angiography, which meanwhile demonstrated a PDA lesion. The patient, after receiving catheter-directed thrombolysis and inferior vena cava filter placement, improved in both neurological and respiratory status.ConclusionFor an uncommon but potentially fatal case with PDA-induced paradoxical embolism causing AIS and PE, early recognition and treatment are vital. Further studies are warranted to determine the optimal management and prognosis of patients with PDA-related embolic events.

Tactics of Surgical Repair of Single Ventricle with Excessive Pulmonary Blood Flow and Obstruction of the Distal Arch of the Aorta in Newborns

Pulmonary artery stenosis with distal aortic arch reconstruction and coarctation of the aorta in newborns is an effective palliative procedure for single ventricle and high pulmonary hypertension on the way to total cavo-pulmonary anastomosis. The aim. To present a case of complex correction of a single ventricle of the heart with tricuspid valve atresia and high pulmonary hypertension, obstruction of the distal aortic arch and coarctation of the aorta, and a final effective Fontan procedure with a good long-term outcome. Case report. On December 15, 2011, a 9-day-old newborn patient M. was admitted for treatment at the Department of Cardiovascular Surgery of Odesa Regional Children’s Clinical Hospital with a diagnosis of: a single ventricle of the heart, transposition of the great arteries, tricuspid atresia, distal arch hypoplasia, coarctation of the aorta, patent ductus arteriosus, high pulmonary hypertension. The first stage of surgical treatment was carried out on December 19, 2011: main pulmonary artery banding, reconstruction of the distal arch of the aorta through modified Amato technique, closure of the patent ductus arteriosus, resection of the coarctation of the aorta and extended end-to-end aortoplasty. At the age of 1 year (December 27, 2012), the second stage was performed: bidirectional Glenn procedure with plastic surgery of bifurcation and right pulmonary artery branch. The third stage was performed at the age of 3 years 11 months (November 19, 2015): Fontan procedure with extracardiac conduit. Conclusions. Early elimination of pulmonary hypertension by pulmonary artery banding ensures the preservation of the pulmonary vascular bed with low resistance, which is a crucial condition for the effective final hemodynamic correction of the single ventricle of the heart – the Fontan procedure. In case of hypoplasia of the distal aortic arch with coarctation in newborns, one of the alternative approaches is the use of modified Amato technique avoiding artificial circulation.

Absent Pulmonary Valve Syndrome with Non-Confluent Branches: Case Report and Literature Review

Absent pulmonary valve syndrome (APVS) is a rare form of congenital heart defect. Among the various variations of this rare anomaly are non-confluent branches of the pulmonary artery (PA) with anomalous origin of one of the PA branches from the patent ductus arteriosus (PDA). Constriction of the PDA in early neonatal age may result in cessation of blood supply to one of the pulmonary artery branches, leading to exclusion of one lung from circulation and gas exchange processes. PDA stenting creates a reliable source of blood flow, and as a first step, an endovascular approach was chosen for treatment. Case report. A newborn (3 kg) with a prenatal diagnosis of APVS and a non-confluent left PA branch filling through PDA was chosen for PDA stenting. Considering the complex anatomy of the heart defect, it was decided to perform PDA stenting to delay total repair until the patient reaches optimal age and weight. Our patient successfully underwent PDA stenting as the first stage, which provided conditions for blood supply to one lung. The second stage involved total repair with good long-term results. Conclusion. APVS treatment may have several options of surgical correction, differing from each other depending on the anatomical features of the defect and the patient’s age. Palliative treatment options help the child transition from the neonatal period and gain optimal weight for total repair.. Analyzing the data from the presented literature, it is noted that prenatal diagnosis is an important stage in determining the urgency and choice of treatment tactics for this defect.

At a crossroads for early medical treatment of persistent patent ductus arteriosus in preterm infants.

Administration of early medical therapy for the patent ductus arteriosus has ebbed and flowed through the years, with a multitude of studies failing to demonstrate a reduction in morbidity or mortality from ductal closure in the preterm population. Concerningly, an increasing number of studies have demonstrated an increase in morbidity, such as bronchopulmonary dysplasia and mortality with the use of early medical therapy to close the ductus. Considering information regarding potential risk without clear benefit in an overall cohort of preterm patients with a patent ductus, use of early medical therapy is increasingly challenging to justify and necessitates studies that will aid in identifying a patient population that would benefit from ductal closure and timing of therapy.

Follow-Up of Secundum ASD, Muscular VSD, or PDA Diagnosed During Neonatal Hospitalization.

The ideal follow-up of neonates who have a secundum atrial septal defect (ASD), muscular ventricular septal defect (VSD), or patent ductus arteriosus (PDA) remains uncertain. Newborns with findings limited to a secundum ASD, muscular VSD, and/or PDA on their neonatal hospitalization discharge echocardiogram and at least one outpatient follow-up echocardiogram performed between 9-1-17 and 9-1-21 were evaluated and patient follow-up assessed through 9-1-23. 95 infants met inclusion criteria. 43 infants had a secundum ASD, 41 had a muscular VSD, and 54 had a PDA at newborn hospital discharge. 39/95 had more than one intracardiac shunt. 56 were discharged from care, 26 were still in follow-up and 13 were lost to recommended follow-up. No patients received intervention during the follow-up period of 2 to 6years. Of the 43 infants with a secundum ASD, 16 (37.2%) had demonstrated closure of the ASD, and 13 (30.2%) were discharged from care with an ASD < 3.5mm in diameter. 3/43 infants with secundum ASD had a defect with a diameter of more than 5mm at their last echocardiogram. No infant discharged from their neonatal hospitalization with a secundum ASD, muscular VSD, or PDA needed any intervention from 2 to 6years of follow-up. Ongoing follow-up with echocardiography of those infants with a secundum ASD is of greater value than of those with muscular VSD or PDA.

Brachiocephalic Vein Duplication: Case Report of a Double Left Brachiocephalic Vein in an Infant.

A patient was delivered at 26weeks (about 6months) gestation via an emergency caesarian section. A patent ductus arteriosus (PDA) and atrial septal defect (ASD) were discovered during an echocardiogram 13days after birth. The patient had catheter-based closure of the PDA and ASD. During a routine echocardiogram to check device placements, it was discovered that there was dilation of the superior vena cava (SVC), and it was suspected that a thrombus was present. Computed tomography angiography (CTA) was completed to better define SVC anatomy and flow acceleration. The CTA demonstrated that there was a double innominate vein.

Patent ductus arteriosus complicated by pulmonary artery aneurysm and chronic thromboembolism: a rare case of ‘double trouble’

Patent ductus arteriosus complicated by pulmonary artery aneurysm and chronic thromboembolism: a rare case of ‘double trouble’

PDA management strategies and pulmonary hypertension in extreme preterm infants with bronchopulmonary dysplasia.

Premature infants are at risk for developing pulmonary hypertension (PH) in the context of bronchopulmonary dysplasia (BPD). Studies suggest a potential link between prolonged patent ductus arteriosus (PDA) exposure and BPD-PH, though management strategies remain controversial. Retrospective echocardiographic evaluation of newborns <29 weeks gestational age with BPD at two distinct centers. Primary objective was to evaluate the relationship between center-specific PDA management strategies (interventional or conservative) and the prevalence of BPD-PH. BPD was defined as oxygen or respiratory support at 36 weeks post-menstrual age (PMA). The presence of PH was defined as either an estimated sPAP of ≥40 mmHg or sEI ≥1.3. Center A has a conservative PDA policy. Center B has a targeted interventional policy. PH rates were similar between sites (21% vs 17%), while rates of PDA treatment was different (7% vs 81). Adjusted models did not demonstrate an association for center or PDA treatment exposure for PH and EI, although infants from Center A had echocardiography evidence of higher systolic eccentricity index (EI; 1.12 ± 0.19 vs 1.06 ± 0.15, p = 0.04). Markers of RV function (TAPSE and RV-FAC) were similar between groups. In preterm infants <29 weeks with BPD, conservative PDA treatment policy was not associated with higher rate of pulmonary hypertension diagnosis. The association between PDA-management approaches and the occurrence of BPD-associated pulmonary vascular disease in premature infants has sparsely been described. We found that a conservative policy, regarding the PDA, was not associated with an increase in pulmonary hypertension diagnosis. We identified that, in patients with BPD, echocardiographic metrics of LV performance were lower.

Randomised crossover study on pulse oximeter readings from different sensors in very preterm infants

ObjectiveIn extremely preterm infants, different target ranges for pulse oximeter saturation (SpO2) may affect mortality and morbidity. Thus, the impact of technical changes potentially affecting measurements should be assessed. We...

Anesthesia in Emergency PDA Stenting with Pulmonary Arteri Atresia

Pulmonary atresia with an intact ventricular septum is a condition that is characterized by a complete obstruction to right ventricular outflow. This condition is fatal if untreated. Ductus arteriosus communicate between pulmonary artery and proximal descending aorta. It is essential in fetal life as a communicating shunt to send blood from right ventricle bypassing the underdeveloped lungs. Ductal patency may be lifesaving in few cyanotic congenital heart diseases to maintain pulmonary blood flow.Case Illustration : We present a case of a 1‑day‑old, 3405 g child who presented to casualty with bluish discoloration. The child had central cyanosis, tachypnea with saturation 80% on 1 L/minute nasal cannula and desaturate to 40%. Transthoracic echocardiography (TTE) revealed intact intraventricular septum with pulmonary atresia and patent ductus arteriosus (PDA). The patient scheduled to an emergency PDA stenting. A heating blanket is installed on the operation table, oxygen saturation with Pulse‑oxymeter, noninvasive blood pressure, end-tidal CO2 monitor, and temperature monitoring were done. Anesthesia induction was performed with 50% oxygen in air, fentanyl 3 µg/kg, ketamine 2 mg/kg, atracurium 0.5 mg/kg and maintained with sevoflurane. The procedure was performed about 20 to 30 minutes. After procedure, oxygen saturation was increased up to 60%. The child was successfully extubated after 24 hours in the Neonatal Intensive Care Unit (NICU) with saturation 92%.Conclusion: PDA stenting is a less invasive palliative procedure for ductal dependent complex congenital heart diseases. Anesthesiologist's role were maintaining arterial saturation and hemodynamics, securing vascular assess, maintaining temperature. It needs thorough understanding of underlying physiology.

Clinical Results of Prosthetic Eye Wear in Patients with Congenital Microphthalmos

Purpose: To report the long-term clinical outcomes of non-surgical treatment involving prosthetic eye wear in patients diagnosed with congenital microphthalmos.Methods: A retrospective review of the medical records of 20 patients was conducted. In total, 21 eyes were diagnosed with congenital microphthalmos between May 2008 and December 2022 at Seoul St. Mary’s Hospital in Korea.Results: This study included 20 patients (12 males and 8 females) with an average age of 4 months at diagnosis. The observed ophthalmic anomalies included two cases of congenital cataract, one of posterior embryotoxon, one of corneo-iris strand, four of iris coloboma, five of central corneal opacity, one of Peter's anomaly, and one of retrobulbar cyst. Accompanying systemic abnormalities were noted, such as lateral ventricle atrophy, corpus callosum atrophy, patent ductus arteriosus, atrial septal defect, and developmental language disorder. Genetic anomalies included anti SS-A/Ro antibody positivity, a 1:100 titer of anti-nuclear antibody, and a PAX6 mutation identified through next-generation sequencing. No specific family histories or pregnancy-related factors were noted. The average follow-up duration was 5.94 years (range: 1 month to 18 years), the average corneal diameter was 4.6 mm, the average axial length was 17.44 mm, the average age for first artificial eye trial was 5.96 years (range: 7 months to 19 years), and the average interval for artificial eye replacement was 22 months (range: 4 months to 5 years and 8 months).Conclusions: Twenty patients with congenital microphthalmos underwent gradual expansion of their prosthetic eyes by regular replacement and size increase without severe complications. This approach led to aesthetically and emotionally positive outcomes for the patients.

Long term follow-up of patients with patent ductus arteriosus after transcatheter closure.

To investigate the concurrent, long-term, and future adverse events and assess the trend of adverse events in pediatric patients with patent ductus arteriosus (PDA) after transcatheter closure. A total of 1590 patients underwent transcatheter PDA closure were enrolled, including 465 patients (median age = 22 months) in the training group and 1125 patients in the validation group. Logistic regression analysis was used to assess independent risk factors associated with concurrent adverse events after closure. The chi-square test was used to evaluate the 5-year follow-up trend. Multivariable logistic regression analysis indicated that low age, female, and high pulmonary end diameter were independent risk factors for concurrent adverse events after closure. For patients without concurrent adverse events and for those who with concurrent adverse events but return to normal, the Chi-square test showed no abnormal results at the 5-year follow-up. Furthermore, the follow-up data of the validation group were not significantly different from those of the training group. The value of long-term follow-up of children may be limited for those who did not have a concurrent adverse event after closure nor for those who had a concurrent adverse event after closure but returned to normal during the 5-year follow-up period. Follow-up monitoring of adverse events tended to be recommended in pediatric patients with PDA after transcatheter closure. However, follow-up in these pediatric patients is expensive and there is a risk of sedation for echocardiography examination frequently. ·Patients who had no concurrent adverse events after closure did not show any abnormality at 5-year follow-up. ·Most of the patients who had concurrent adverse events after closure returned to normal at 5-year follow-up. The value of long-term follow-up may be limited for the above patients after transcatheter PDA closure.